Lethally aggressive multiply recurrent adamantinomatous craniopharyngioma without histological malignancy: illustrative cases.

Multiply recurrent ACP with a high Ki-67 LI can follow a rapidly lethal course despite benign histology. Early recognition of this aggressive phenotype should prompt short-interval MRI surveillance and timely, goal-directed salvage focused on proactive cyst control, hypothalamus-sparing strategies, and carefully selected reirradiation. When standard options fail, enrollment in clinical trials of emerging biological therapies, such as IL-6 blockade, intracystic peginterferon-α2a, and EGFR-axis treatments, should be considered. https://thejns.org/doi/10.3171/CASE25840.